A Case of Overlap Syndrome of Systemic Sclerosis and Cryoglobulinemic Vasculitis With Central Nervous System Involvement

Jung, Youjin; Lee, Eunyoung Emily; Yeo, Jina; Kim, Mi Hyeon; Choi, Se Rim; Song, Yeong‐Wook

doi:10.4078/jrd.2021.28.4.242

Cited by 3 publications

(4 citation statements)

References 16 publications

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“…Our patient had normal CSF findings initially and later had a high glucose with normal protein and a high IgG index. The CNS findings do not correlate well and have been found to be normal initially even with subsequent findings of CNS vasculitis in patients [ 4 , 5 ]. The pathophysiological mechanism of vasculopathy and initial imaging findings remain unclear but are most likely secondary to vasculitis.…”

Section: Discussionmentioning

confidence: 99%

“…Differential diagnosis includes posterior reversible encephalopathy syndrome (PRES) or hypertensive encephalopathy in the setting of renal failure. PRES has vasogenic edema on brain imaging with dark lesions on diffusion-weighted imaging (DWI) [ 5 ]. In our patient, the MRI brain showed global cerebral edema on fluid-attenuated inversion recovery (FLAIR), with minimal changes on DWI suggestive that this was not an infarction.…”

Section: Discussionmentioning

confidence: 99%

“…Although the frequency of neurological involvement is uncertain, it is estimated to occur in 50-60% of cases [ 2 ]. While peripheral nervous system involvement is more commonly seen, central nervous system (CNS) involvement is quite rare, approximately 0-3% [ 3 , 4 , 5 ]. CNS manifestations include transient dysarthria, confusion, and hemiplegia.…”

Section: Introductionmentioning

confidence: 99%

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A Case of Cryoglobulinemia With Central Nervous System Involvement

Noor,

Ogunleye,

Rahim

et al. 2024

Cureus

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Cryoglobulinemia may result in small-to-medium vessel vasculitis. Central nervous system (CNS) involvement is rare, and presentation may range from stroke/transient ischemic attack, reversible ischemic neurological deficits, to encephalopathic syndromes. This is a rare case discussing cryoglobulinemia with CNS involvement. A 56-year-old female with a history of cryoglobulinemia was found unresponsive to verbal and physical stimuli. She was admitted to the intensive care unit. CT head without contrast showed diffuse cerebral edema and mass effect in the right cerebral hemisphere causing right to left midline shift, brainstem infarct, hemorrhage in the right lateral ventricle, and obstruction of the fourth ventricle. The patient was managed with hypertonic saline, external ventricular drain (EVD) placement, and high-dose steroids, which led to an improvement in her condition. In conclusion, testing for cryoglobulins and serologic tests for hepatitis C should be considered in syndromes of cerebral ischemia or infarction without an obvious cause, especially in young individuals since encephalopathy may be reversible. Cryoglobulinemia with CNS manifestations may be associated with purpura, high RF, and low C4. The treatment can be a combination of steroids, immunosuppressants, plasmapheresis, and rituximab. Cyclophosphamide may also be considered as adjunctive therapy to corticosteroids in rapidly progressive severe neurological complications. Further research for treatment standards in nonviral cryoglobulinemia is needed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A Case of Cryoglobulinemia With Central Nervous System Involvement

Noor,

Ogunleye,

Rahim

et al. 2024

Cureus

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“…Interstitial lung diseases (ILDs) are a type of diffuse parenchymal lung disease that is marked by interstitial involvement induced by inflammation and fibrosis ( 1 , 2 ). Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia that has no recognized etiology.…”

Section: Introductionmentioning

confidence: 99%

Efficacy and Safety of Nintedanib in Patients with Interstitial Lung Disease with or without Systemic Sclerosis: A Meta-Analysis

Lee,

Song

2023

ijph

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Background: Nintedanib is a potent intracellular inhibitor of tyrosine kinases and modulates the pathways involved in the development of fibrosis. We assessed nintedanib efficacy and safety in interstitial lung disease (ILD) patients. Methods: We searched MEDLINE, EMBASE, and the Cochrane Controlled Trials Register to identify available articles (up to April 2022). We conducted a meta-analysis of randomized controlled trials (RCTs) examining nintedanib efficacy and safety in patients with ILD with or without systemic sclerosis (SSc). Results: Meta-analysis of five RCTs including 2,470 patients with ILD (1,343 nintedanib group and 1,127 controls) revealed that the annual rate of change in forced vital capacity (FVC) was significantly lower in the ILD group than in the control group (standardized mean difference [SMD] = 0.336; 95% confidence interval (CI) = 0.256–0.416, P<0.001). Stratification by disease type showed a low annual rate of change in FVC in patients with and without SSc (SMD = 0.389, 95% CI=0.294–0.478, P<0.001; SMD=0.177, 95% CI=0.013–0.340, P<0.00). The incidence of serious adverse events did not differ between the nintedanib and placebo groups; however, adverse events (AEs) and withdrawals due to AEs were significantly higher in the nintedanib group than in the placebo group (SMD =2.365, 95% CI=1.673-3.343, P<0.001; SMD =1.740, 95% CI= 1.385-2.185, P<0.001). Conclusion: Nintedanib is effective for ILD with or without SSc. However, it increased the incidence of AEs and withdrawals due to AEs.

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Standardized mortality ratios in systemic sclerosis: a meta-analysis assessing overall and sex- and disease subtype-specific differences

Lee,

Song

2023

Z Rheumatol

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A Case of Overlap Syndrome of Systemic Sclerosis and Cryoglobulinemic Vasculitis With Central Nervous System Involvement

Cited by 3 publications

References 16 publications

A Case of Cryoglobulinemia With Central Nervous System Involvement

A Case of Cryoglobulinemia With Central Nervous System Involvement

Efficacy and Safety of Nintedanib in Patients with Interstitial Lung Disease with or without Systemic Sclerosis: A Meta-Analysis

Standardized mortality ratios in systemic sclerosis: a meta-analysis assessing overall and sex- and disease subtype-specific differences

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